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Intercalated discs and arrhythmogenic cardiomyopathy

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Abstract
Heart tissue is subjected to high mechanical stress. Different junctional complexes exist within the intercalated disc (ID) at the site of end-to-end contacts between cardiomyocytes. These junctions are essential for adhesive integrity, morphogenesis, differentiation, and maintenance of cardiac tissue. Recent findings of molecular interactions among intercellular adhesion molecules, gap junctions, and the voltage-gated sodium channel complex suggest that IDs should be considered an organelle in which macromolecular complexes interact specifically to maintain cardiac structure and cardiomyocyte synchrony. It is within this organelle that most of the mutated proteins involved in arrhythmogenic right ventricular cardiomyopathy (ARVC) reside. This inherited cardiomyopathy is characterized by both structural and electric abnormalities of the heart, particularly in young people and athletes. This review highlights recent advances in understanding the link between ID alterations and the molecular genetics and pathogenesis of ARVC.
Keywords
NAXOS-DISEASE, ADHERENS JUNCTIONS, ADHERING JUNCTIONS, DILATED CARDIOMYOPATHY, AREA-COMPOSITA, SODIUM CURRENT DEFICIT, RYANODINE RECEPTOR GENE, ALPHA-T-CATENIN, HEART-MUSCLE CELLS, RIGHT-VENTRICULAR CARDIOMYOPATHY, molecular biology, mutation, cell adhesion molecules, arrhythmogenic right ventricular dysplasia-cardiomyopathy, arrhythmias

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Citation

Please use this url to cite or link to this publication:

Chicago
Rampazzo, Alessandra, Martina Calore, Jolanda van Hengel, and Frans Van Roy. 2014. “Intercalated Discs and Arrhythmogenic Cardiomyopathy.” Circulation-cardiovascular Genetics 7 (6): 930–940.
APA
Rampazzo, A., Calore, M., van Hengel, J., & Van Roy, F. (2014). Intercalated discs and arrhythmogenic cardiomyopathy. CIRCULATION-CARDIOVASCULAR GENETICS, 7(6), 930–940.
Vancouver
1.
Rampazzo A, Calore M, van Hengel J, Van Roy F. Intercalated discs and arrhythmogenic cardiomyopathy. CIRCULATION-CARDIOVASCULAR GENETICS. 2014;7(6):930–40.
MLA
Rampazzo, Alessandra et al. “Intercalated Discs and Arrhythmogenic Cardiomyopathy.” CIRCULATION-CARDIOVASCULAR GENETICS 7.6 (2014): 930–940. Print.
@article{5862803,
  abstract     = {Heart tissue is subjected to high mechanical stress. Different junctional complexes exist within the intercalated disc (ID) at the site of end-to-end contacts between cardiomyocytes. These junctions are essential for adhesive integrity, morphogenesis, differentiation, and maintenance of cardiac tissue. Recent findings of molecular interactions among intercellular adhesion molecules, gap junctions, and the voltage-gated sodium channel complex suggest that IDs should be considered an organelle in which macromolecular complexes interact specifically to maintain cardiac structure and cardiomyocyte synchrony. It is within this organelle that most of the mutated proteins involved in arrhythmogenic right ventricular cardiomyopathy (ARVC) reside. This inherited cardiomyopathy is characterized by both structural and electric abnormalities of the heart, particularly in young people and athletes. This review highlights recent advances in understanding the link between ID alterations and the molecular genetics and pathogenesis of ARVC.},
  author       = {Rampazzo, Alessandra and Calore, Martina and van Hengel, Jolanda and Van Roy, Frans},
  issn         = {1942-325X},
  journal      = {CIRCULATION-CARDIOVASCULAR GENETICS},
  keywords     = {NAXOS-DISEASE,ADHERENS JUNCTIONS,ADHERING JUNCTIONS,DILATED CARDIOMYOPATHY,AREA-COMPOSITA,SODIUM CURRENT DEFICIT,RYANODINE RECEPTOR GENE,ALPHA-T-CATENIN,HEART-MUSCLE CELLS,RIGHT-VENTRICULAR CARDIOMYOPATHY,molecular biology,mutation,cell adhesion molecules,arrhythmogenic right ventricular dysplasia-cardiomyopathy,arrhythmias},
  language     = {eng},
  number       = {6},
  pages        = {930--940},
  title        = {Intercalated discs and arrhythmogenic cardiomyopathy},
  url          = {http://dx.doi.org/10.1161/CIRCGENETICS.114.000645},
  volume       = {7},
  year         = {2014},
}

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